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October 1987

Sézary-like Syndrome in a 10-Year-Old Girl With Serologic Evidence of Human T-Cell Lymphotropic Virus Type I Infection

Author Affiliations

From the Departments of Dermatology (Dr Ikai) and Internal Medicine (Dr Uchiyama), Faculty of Medicine, and the Department of Cytochemistry (Dr Maeda), Chest Disease Research Institute, Kyoto University, and the Department of Dermatology, Hamamatsu University School of Medicine (Dr Takigawa), Japan.

Arch Dermatol. 1987;123(10):1351-1355. doi:10.1001/archderm.1987.01660340113031

• A 10-year-old girl with a Sézary-like syndrome is described. At age 6 months, her skin involvement began as psoriatic plaques. Generalized erythema, lymphadenopathy, and pruritus developed into erythroderma. Her general condition has been good in spite of severe pruritus and erythroderma. Atypical cells were found in the peripheral blood in numbers of 2500 mm3 and were present in the dermis of a skin biopsy specimen. When examined by electron microscopy, these cells showed lobulated or indented nuclei and were similar to Sézary cells. The patient showed a high titer (1:1280) of antihuman T-cell lymphotropic virus type I antibody. Her mother was also positive with a titer of 1:40 and seems to be a healthy carrier.

(Arch Dermatol 1987;123:1351-1355)

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